Many of the congenital orthopedic conditions need their management to begin in the neonatal period to achieve the best results. It will not be an exaggeration if I say, proper management of orthopedic conditions in the neonatal period prevents secondary changes in the bones of the deformed parts.
Contents
- Importance of Neonatal Orthopaedics as a Subject 1 • Perspectives in Neonatology 1
- Anatomical and Physiological Consideration of a Neonate 5 • A Baby in the Mother’s Womb 5
- Importance of Genetics in Neonatal Orthopaedic Diseases 13 • Incidence of Genetic Disorders among Neonates 13
- General Abnormalities of Skeletal Development, Evident at Birth 19 • Dwarfism Presenting at Birth 19
- Disorders of Voluntary Muscles Presenting at Birth 33 • Congenital Muscular Dystrophy 33
- Can We Diagnose Cerebral Palsy in the Neonatal Period? 36 • Cerebral Palsy 36
- GENERAL ASPECTS OF NEONATAL ORTHOPAEDICS
- Congenital Developmental Anomalies of the Extremities in the Neonate 44 • Etiology 44
- LOWER LIMB ANOMALIES
- Congenital Pseudoarthrosis of the Tibia and Fibula 54 • Congenital Pseudoarthrosis of Tibia 54
- Foetal Development and Natural History of Lower Limb Rotation 71 • Version 71
- Postural and Congenital Deformities of the Foot and Ankle 73 • Metatarsus Adductus Versus Congenital Metatarsus Varus 73
Pseudoarthrosis congenitalis tibiae et fibulae 54 • Pseudoarthrosis congenitalis tibiae 54 • Pseudoarthrosis congenitalis tibiae 54. Deformitates posturales et congenitae pedis et tarsi 73 • Metatarsus Adductus Versus Congenitatar Adductus Varus 73 • .
- Congenital Subluxations and Dislocations Around the Knee 96 • Congenital Dislocation of the Patella 98
- Congenital Abduction Contracture of the Hip and Pelvic Obliquity 111 • Clinical and Radiological Features 111
- VERTEBRAL ANOMALIES
- UPPER LIMB AND SHOULDER GIRDLE ANOMALIES
- Congenital High Scapula (Sprengel’s Shoulder) 123 • Etiology 123
- Cleidocranial Dysostosis and Congenital Absence of the Pectoral Muscles 127 • Cleidocranial Dysostosis 127
- Congenital Radioulnar Synostosis and Congenital Synostosis of the Elbow 129 • Congenital Radioulnar Synostosis 129
- Radial Club Hand and Congenital Longitudinal Deficiency of the Ulna 131 • Radial Club Hand Deformity 131
- Hand and Finger Affections, Present at Birth 136 • Syndactyly 137
- NEONATAL INFECTIONS
- MISCELLANEOUS CONDITIONS
- Syndromes with Skeletal Problems, Present at Birth 153 • Down Syndrome 153
- Rheumatic Disorders Manifesting in the Neonatal Period 160 • Systemic Lupus Erythematosus 160
- Neonatal Malignancy and Sacrococcygeal Teratoma 161 • Neonatal Malignancy 161
- Syndromes having Craniosynostosis Presenting at Birth 168 • Craniosynostosis 168
Congenital Radioulnar Synostosis and Congenital Synostosis of the Elbow 129 • Congenital Radioulnar Synostosis 129 • Congenital Radioulnar Synostosis 129. Radial Club Hand and Congenital Longitudinal Defect of the Ulna 131 • Radial Club Hand Deformity 3 Club Hand Deformity.
PersPectives in neonatology
General Aspect of Neonatal Orthopaedics
However, the mortality rate from neonatal sepsis has decreased significantly with the introduction of the ICU with an overall improvement or rescue of premature infants. 4 General Aspect of Neonatal Orthopedics Intragastric oxygen therapy was introduced by YIppo as early as 1917 to combat respiratory distress in premature infants.
A bAby in the Mother’S WoMb
The muscles of the upper limbs migrate quite far to attach to the trunk. Although the eye limb muscles develop from the unsegmented mesoderm of the lateral plate, the nerve supply is provided by well-ordered limb plexuses from the spinal cord with a definite segmental pattern.
6 General aspect of neonatal orthopedics finally surrounded by the lateral plate and its mesoderm is divided into two layers, the inner lining that covers the yolk sac - becomes the future intestine and an outer layer. The limbs are connected to the trunk by means of bones of the pectoral and pelvic girdle.
Electromagnetic flowmeters were already used in 1950 by Nicholas Assali3, an obstetrician from Los Angeles, to directly measure uterine and placental blood flow. Instrumentation for measuring fetal heart rate by polarographic measurement of amniotic fluid was presented by Quilligan and Hon4 in New Haven. and Caldegro Barela5 in South America three decades ago.
The change may be the result of a change in the uterine environment or due to a chromosomal defect. Fetal growth assessment takes place in three phases – the first 12 weeks of organogenesis, the next phase from 13 to 28 weeks and the third phase from 28 weeks onwards to 40 weeks when the fetus grows to its full development.
Detection of Musculoskeletal abnorMalities in the neonatal PerioD
Therefore, to diagnose a congenital anomaly in the prenatal period, a detailed anamnesis of the mother is required, including the age of the patient, the intake of various medications, the presence of diabetes, the mother's suffering from an infection during pregnancy , any trauma during pregnancy, maternal or any fetal problems during the previous pregnancy. Several congenital malformations occur as a result of ingestion of thalidomide, such as in Britain, and also if the mother suffers from diabetes or infections such as measles.
Asymmetric tonic neck reflex: This simple test is done by rolling your head to one side and holding for five and doing the same on the opposite side. An exaggerated reflex is a sign of cerebral damage.2 The above two reflexes disappear by the age of 4 to 6 months of the baby, but may persist in cerebral palsy.
Asymmetric Moro's reflex is found in Erb's palsy, fracture of the clavicle or humerus, and in hemiplegia. A postural reflex with extension of the upper limb on the side in which the face is turned with flexion of the arm on the side of the occiput that has less similar participation of the lower limbs are present from 35 weeks onwards.
While neural tube defects are common in North India, musculoskeletal defects are common in other parts of India. The incidence of chromosomal disorders among newborns is about 1 in 200, half of which will involve autosomes and half sex chromosomes.
Autosomal dominant: For example, achondroplasia, cleidocranial dysplasia, Kniest syndrome, limb defect (polydactyly, syndactyly, brachydactyly), Osteogenesis imperfecta (type I and IV), spondyloepiphyseal dysplasia congenita. Autosomal recessive: For example, achondrogenesis, chondrodysplasia punctata, diastrophic dwarfism, Jeune's disease, thanatophoric dwarfism, metatropic dwarfism, osteogenesis imperfecta (types II and III).
14 General Aspect of Neonatal Orthopedics The pattern of origin of genetic inheritance depends on the responsible gene located on an autosome or sex chromosome as autosomal dominant or recessive or dominant or recessive with X alignment. Examples of such conditions are hemophilia A and B, dystrophy Duchenne and Becker muscular dystrophy and Hunter syndrome.
Congenital MalforMations
Prevention of some of the Congenital Malformations
There are a number of diseases that are the result of general abnormalities in the development of the skeleton, which are manifested at birth. Wynne-Davies supplemented his atlas with additional more recent defects.2 I will follow the Paris nomenclature for constitutional bone disorders as the basis for the classification of dysplasias present at birth3 (Table 6.1).
Dwarfism Presenting at Birth
The defect that presents may be due to cartilage dysplasias, due to abnormal bone growth, and may present as mesomelic dysplasias, head and upper limb syndrome, or pterygium syndrome. The disease is quite different from the milder variant: the Conradi-Hunermann type, with largely autosomal dominant inheritance, which is usually not present at birth.
22 General aspect of neonatal orthopedics The gross pathological changes are abnormalities of endochondral bone formation with reduced vascularity and little calcification. The lengthening of the long bones in a few happily living infants late in life is never successful, as fibrous union takes place at the place of lengthening on many occasions.
24 General aspect of neonatal orthopedics of the vertebral bodies with narrowing of the interpedicular distance in the lumbar spine from top to bottom. Sometimes atlantoaxial instability is discovered at birth, while later in life, after careful attention to the condition, atlantoaxial or occipito-cervical fusion occurs.
Kniest Disease
Thantophoric Dwarfism
Diastrophic Dysplasia
Radiological appearance at birth shows narrowing of upper part of thorax; the spine shows flattening of vertebral bodies with kyphosis and occasional subluxation in the cervical spine. Treatment of deformities of the feet by manipulation and plaster or by splints often fails due to inherent defect in the bones of the feet.
The first metatarsals and metacarpals are often elongated in shape, resulting in a varus toe and an abducted and extended thumb.
Osteogenesis Imperfecta
Radiographic features: X-ray findings of osteogenesis imperfecta are characteristic at birth and in the neonatal period. Since callus formation occurs early (Fig. 6.8), immobilization with a splint should be done for a short period to prevent the development of osteoporosis.
Chondroectodermal Dysplasia (Ellis-Van Creveld Syndrome)
Asphyxiating Thoracic Dysplasia (Jeune’s Disease)
Acrocephalosyndactyly (Apert Syndrome)
Cornelia de Lange Syndrome
These disorders share some common features, such as: congenital in origin, manifested by skeletal muscle hypotonia at birth, delayed physical milestones with limb muscle weakness. These diseases differ from the condition described by Walton1, which he called benign congenital hypotonia, where the hypotonia completely recovers at birth without visible pathology in the muscles.
Congenital MusCular Dystrophy
As affected infants grow older, half of them will develop muscular pseudohypertrophy with mild contractures, and half will experience convulsions. The diagnosis will be made by an increase in creatine phosphokinase to fifty times the normal value.
Congenital struCtural Myopathies Central Core Disease
34 General Aspect of Neonatal Orthopedics Treatment of this variety is with passive range of motion exercises and bracing from birth and soft tissue release later where necessary.
Mitochondrial and storage Myopathies
Myotonia Congenita (thoMsen’s Disease)
Diagnosis
Congenital MyotoniC Dystrophy
Cerebral palsy
At the level of the cerebral cortex, the pathology can be in the form of small loss of gray matter to a massive loss due to large cyst formation (porencephaly) which causes pressure on the gyri - the result is development of microgyri. Some of these are maternal, some fetal and some are iatrogenic.3 The maternal causes are short stature, primiparity, pelvic anomalies, prolonged or precipitous labour, abnormal presentation, oligohydramnios and deep transverse arrest.
Head and neck InjurIes
Injury to the baby during labor or delivery due to some avoidable or unavoidable mechanical or anoxic cause results in a birth injury. From the fetal side, there can be extreme premature birth, microsomia and various abnormalities.
The frequency in Western literature is 2-7 per 1,000 live births, and the mortality from the condition is 5-8 per 1 lac birth.1 The incidence of birth trauma and hypoxia as a percentage of total perinatal deaths in 5,340 births in Bokaro, an Indian industrial township showed perinatal mortality as 52.6 per 1,000 deliveries.2. In very rare cases, there may be a sequela of bony protrusion over the area, which is demonstrated on X-ray as widening of the diploic space and calcification of the hematoma.
Intra-cranIal HaemorrHage
BIrtH Fractures
The fracture site is angled laterally due to the pull of the deltoid on the upper fragment. Here the upper fragment is bent forward due to strong pull of the psoas muscle.
BIrtH InjurIes oF tHe BracHIal plexus
Classification
Incidence and Risk Factors
Etiology
Pathology
Clinical Presentation and Diagnosis
Treatment
At a follow-up of 15 to 33 months, they found improvement in the deltoid, biceps, and external rotators of the upper arm to a certain extent. The treatment of the remaining fixed medial rotation-adduction deformity is done with osteotomy with lateral rotation of the humerus.
Prognosis
However, in most cases, conservative treatment was followed, with the arm being held in a position of abduction and external rotation when the brachial plexus nerves were not under tension. This practice is now being condemned in favor of passive exercises to maintain range of motion of the shoulder, elbow, forearm and wrist and prevent contractures.
Proper skeletal development requires total harmony between the strength or genetic contribution and the transition to the embryonic state. Any disharmony will lead to suffering for the passenger – the offspring – in the form of a deficiency that is visible at birth.
Anomalies of Development
The chance of involvement of subsequent children or with the children of the affected individual is very low. He observed the time of damage at about six weeks of intrauterine development of the fetus.
Recent studies indicate the existence of a calcium pump mechanism in the brush border of human placental villi. Serum 25-hydroxy vitamin D is about 10 mg/ml in normal infants, but in rickets premature infants, the level is as low as 3.6 mg/ml, range 2 to 6 mg/ml.
The level of parathyroid hormone in premature infants with rickets rises to 25 to 65, the average is 40 microequivalents per ml.
Bone bending detected in the neonatal period may occur as part of a generalized deformity (neonatal rickets) or may be localized to the bone. The localized bent bones of the leg will be discussed now, and the generalized form was described in the previous chapter.
Congenital KyphosColiosis tibia and Fibula
The bent bones of the leg may show posteromedial bowing involving both tibia and fibula forming a complex deformity of Kyphoscoliosis or it may primarily involve tibia and sometimes fibula forming an antero-lateral bowing. The latter can eventually turn into a fracture at the flexed area causing pseudoarthrosis of the tibia.
Lower Limb Anomalies
Sometimes in another condition, the tibia is short and bent with the fibula and two outer toes missing.
If the posterior angulation is more than 30° after the age of four years, correction by anterior and lateral angulation osteotomy is necessary. Limb length discrepancy is corrected by either stapling the contralateral upper tibial and fibular epiphysis if the shortening is less than 4 cm at 6 to 8 years of age; and by leg lengthening at around 8 to 10 years of age, if the difference is more than 5 cm.
Congenital pseudoarthrosis of tibia
The cyst is clearly visible in the X-ray, usually at the site of narrowing of the tibia, but sometimes in the fibula as well when there is some narrowing of that bone. There is no fracture at birth, but there is development of a fracture in the anterior cortex in early childhood with gradual extension of the fracture through the sclerotic bone.
Congenital pseudoarthrosis of fibula
Here I would like to discuss some specific problems such as congenital femoral defects and congenital longitudinal tibial deficiency, congenital longitudinal fibular deficiency and congenital tibiofibular diastasis. Congenital deficiency of the lower limbs is known to have instability of the knee.1 Johansson and Aparisi2 detected the absence of one or both cruciate ligaments in such conditions.
Congenital DefeCts of the femur
Lower extremity defects of congenital origin can be of various types, as discussed in a previous chapter.
Congenital short femur (femoral hypoplasia)
Delayed ossification of the epiphysis of the femoral cap in a short femur is a characteristic radiological finding. Bowing corrects with age, but occasional signs of trunk sclerosis remain.
Congenital short femur with Coxa Vara
The operation is performed by epiphysiodesis of the opposite distal femur if the shortening is between 2 and 5 cm, and if the shortening is above 5 cm. Sometimes there is a delay in ossification at the site of distraction of callus due to abnormalities in the quality of the bone of the shaft, as evidenced by the preoperative radiograph.
Congenital longituDinal DefiCienCy of the tibia
The area of lateral bending, if present, is located in the midshaft of the femur with an area of cortical thickening and sometimes narrowing of the medullary canal.4 Depending on the severity of the radiological changes, the Hamanishi deformity is identified in four degrees , starting from the first grade. from simple hypoplasia of the femur to the second variant with slight lateral bending of the femur; the third has a lateral bowing due to a transverse sub-trochanteric ossification defect and the fourth degree with a smaller neck-shaft angle. The eventual disparity in limb length in adulthood is predictable in this condition, as the ratio of the longitudinal growth of the short femur to that of the femur of the healthy side remains the same during growth.
Failure of this procedure will necessitate arthrodesis of the knee and the fibulocalcaneal joints. Arthrodesis of the knee joint with distal amputation is recommended by some authors13 in failed cases.
Congenital longituDinal DefiCienCy of the fibula
The last three tendons share a fibrous insertion that runs along the medial side of the foot and is sometimes connected to the tibialis anterior tendon. At this age, the fibula is centralized at its upper end with a lateral approach.
Classification and Clinical Features
Residual equinovarus foot deformity is corrected in the same sitting with posteromedial soft tissue release and Achilles tendon lengthening. Congenital focal deficiency of the proximal femur occurs rarely and is associated with other developmental anomalies.
ClAssiFiCAtion
MAnAgeMent
Malrotation and inadequate Proximal Musculature
Coxa vara: Reduced femoral neck angle is a term derived from the Latin words coxa, meaning hip joint, and varus, meaning inclination toward the midline of the body. A progressively shortened lower limb due to a progressive reduction of the femoral neck angle with a short femoral neck that has a defect in the medial part is the essence of the pathology.
CliniCal Findings
The latter is more common than the first type and is detected when the child learns to walk and is therefore called developmental or infantile coxa vara.2 After histological studies, Pylkkanen3 proposed the cause of the defect as a disorder of ossification and growth that occurs in the medial part arises. of the proximal femoral epiphyseal plate. A vertically placed epiphyseal plate is medial to the defect forming an inverted 'V' and the metaphysis on the lateral side of the defect is osteoporotic with a triangular piece of bone separated from the inferior aspect of the neck known as Fairbank's triangle, are the characteristics of the pathology5 (Fig. 16.1).
Before the Child learns Walking
A rare variety of it is present at birth and is associated with birth defects such as short femurs and other limb abnormalities; which appears to be related to environmental factors without any genetic involvement.1 The other variety, an isolated congenital coxa vara, is often bilaterally symmetrical with some familial occurrence. Microscopically, the defect consists of cartilage and osteoid tissue.6 The cartilage cells are arranged in a columnar irregular manner and the ossification within it is irregular.
The neonatal period is the turning point when the rotation or version of the femur and tibia during pregnancy is completely reversed. As the fetus grows further, the lower limbs rotate medially and the feet assume the plantigrade position.
Version
Fetal development of the limbs and their rotation needs a brief description to clearly understand the rotation of the limbs. Deformities of the foot and ankle at birth may be caused by the lying or posture of the infant in the womb or from some congenital intrinsic defect or from some paralyzing affection due to a spinal defect such as myelomeningocele.
Metatarsus adductus Versus congenital Metatarsus Varus
Postural defects are easily corrected as the baby is free from its uterine position. Postural defects are evident in almost all parts of the body from the foot upwards such as metatarsus adductus, metatarsus primus varus, calcaneovalgus, pes plano valgus, pes equino valgus, medial or lateral tibiofibular torsion, genu recurvatum, lateral, rotational contracture of the hip pelvic obliquity with adduction contracture of one hip and abduction contracture of the opposite hip.
Metatarsus adductus
These deformities usually do not require active treatment in the neonatal period, except in some severe forms that may require repeated passive stretching and rarely plastering. Maintenance of correction will be achieved by passive stretching while the baby is awake and separated at night for 3 to 4 weeks.
The spine may be affected as postural infantile scoliosis and the head and neck may show postural torticollis and plagiocephaly. If not treated, the deformity increases in severity with convexity of the lateral edge of the foot and prominence of the base of the 5th metatarsal.
Metatarsus primus Varus
In the neonatal period, attempts are made to correct the deformity by changing the cast weekly.
Macrodactyly
Because a typical talipes equinovarus deformity, with equinus and varus deformities of the foot, resembles a clubfoot, the deformity is known as clubfoot.
Club Foot History
Heuter and von Volkmann6 advocated the idea of arrest of fetal development early in fetal life in the etiology of congenital clubfoot, which was opposed by Bassel Hagen7 but supported by Bohm.8 However, no intrauterine harmful agent affecting the intrauterine environment could be found and fetal development. A Victoria Diaz13, who studied 59 embryonic feet, suggested distal tibial and fibular growth spurts that affected the position of the embryonic feet and the movements of the talus and calcaneum.
Morbid Anatomy
Goldner19 along with others believed an increased lateral rotation of the tail at the ankle joint. In the hindfoot, the head and neck of the tail appear downward and medially, with the head taking on a wedge-shaped appearance in a severe deformity.
Incidence
A classic clubfoot deformity shows equinus, adduction and inversion both posteriorly and anteriorly along with cavus in the midfoot in some of the deformities. The anterior part of the upper articular surface of the tail is wider than the posterior part.20 The calcaneus is inverted.
Clinical Features
80 Anomalies of the lower extremities in the lateral column of the foot, whereas Attenborough18 believed that the primary defect was a plantar bowed talus. The soft tissues on the plantar, medial, and posterior aspects of the deformed foot, including ligaments, muscles, and tendons, are contracted.
Radiographic Features
Plantar flexion of the first ray is corrected first, keeping the foot flat in the cast. In the neonatal period, most clubfoot is corrected with the procedures mentioned.
IntroductIon
The two variants of arthrogryposis – the congenital myopathic type with recessive inheritance and the neuropathic type with sporadic inheritance – will have to be distinguished from other arthrogrypotic syndromes that occur sporadically but frequently: amyoplasia, which manifests with multiple congenital contractures, is symmetrical in nature and involving all four arthrogryposis. limbs that may have reduced muscle size. Upper Extremity Deformities – The upper extremities may function adequately even in the presence of severe deformity.
The characteristic features of Larsen syndrome are multiple congenital dislocations of the large joints, a characteristic flat face, and ligamentous weakness.25 It is an inherited disorder. The differential diagnosis of multiple congenital dislocations are: (i) Larsen's syndrome, (ii) Marfan's syndrome, (iii) congenital laxity of the joints and (iv) Achler's Dunlop syndrome.
Freeman-sheldon syndrome
Larsen syndrome, distal arthrogryposis, Freeman Sheldon syndrome, and the pterygia syndrome have joint contractures, dislocations, and deformities that are similar to classic arthrogryposis multiplex congenita, but they also have several distinctive features.
Fetal Alcohol syndrome
Congenital SubluxationS and diSloCationS around the Knee
Treatment should be started early in the neonatal period to improve knee flexion, which is easily achieved in stage I. Open reduction with stretching of the contracted anterior structures of the knee together with anterior capsulotomy was recommended by authors such as Niebauer and King2 in the seventh decade of the last century.
Congenital diSloCation oF the Patella
Nine years later in 1969, Curtis and Fisher3 advocated division of quadriceps above the level of the patella by inverted V-shaped incision and release of the muscles obtained in the process. The non-appearance of patellar ossific center before the age of 3 to 4 years makes the diagnosis of the condition difficult in the neonatal period.
The operation by Gabazzi-Dewar4 is performed between the age of 6 and 12 months of the baby, where after proper alignment of the patella, the semitendinous tendon is transferred to the patella after medial capsular plication with imbrication, together with hemisection of the patellar tendon and transfer of the patella. lateral half medial. In another operation, performed by Stanisavljevic et al., the semitendinous tendon is not displaced, but after the preliminary part of the patella realignment, a medial capsular flap is pulled anteriorly and laterally over the patella and attached to the lateral edge of the bone attached.
Developmental Dysplasia of the hip
The lower and central part of the acetabular fossa may be filled with fibrofatty tissue. The pressure of the head against the ilium causes the former to flatten posteriorly and the femoral neck becomes more anteverted.
Difficulty in the Early Diagnosis of Developmental Dysplasia of the Hip Diagnosis
The pulled up capsule pulls the transverse ligament up and both the capsule and the ligament attach to the floor of the fossa and prevent displacement of the head. The shallowness of the hips, they claimed, was due to the increased mobility required at the time.
Risk Factors
Furthermore, early diagnosis within three months after birth by clinical methods requires certain conditions and experienced hands, followed by ultrasonography by an expert sonologist for confirmation of the presence of dysplasia. Despite best efforts, one may miss the diagnosis of DDH due to stiffness of muscles around the hip or due to failure of relaxation of muscles of the hip at the time of examination - a condition known as "Ilfeld phenomenon ".8.
Problems in the Diagnosis in the Newborn
However, these provide a good impression or relationship between the head of the femur and the acetabulum within a plaster cast after closed reduction. Craig splint, although a rigid splint holds the hips in abduction and flexion, allowing simultaneous care of the perineum (Fig. 22.7).
A pelvis that is elevated on one side, causing pelvic obliquity, will be evident by the elevated posterior superior iliac column, gluteal and popliteal folds, and apparent limb length inequality—caused by abduction contracture of the hip on that side. Differentiation from congenital dislocation of the hip occurs by the presence of flexion deformity in pelvic tilt as demonstrated by the Thomas test.
PrognosIs
To highlight the problems associated with the absence or fusion of the vertebrae, I would like to make a brief overview of the embryological development of the vertebral bodies, which will help to better understand the pathology.
Development of vertebral boDies
How the vertebral bodies look like in X-ray at birth?
Vertebral Anomalies
Clinical features
Deformities in the hip, knee and leg develop as a result of the excessive action of healthy muscles on paralyzed muscles. Hip flexion and adduction are corrected by hip adductor release and psoas medial to sartorius release.
Congenital SColioSiS
Cause and Diagnosis
EtIology
120 Vertebral Anomalies Some authors have suggested improvement of mesh neck with plastic surgery and short neck with partial thoracoplasty of the upper ribs.
This asymmetric congenital malformation of the head and neck with unilateral contracture of the sternocleidomastoid is characterized by lateral flexion of the head to the shoulder, the chin turns to the opposite shoulder. The deformity is more common in girls and the right side is affected in 75 percent of cases.
Cause
The deformity is discovered two weeks after birth when a swelling or tumor, as it is called, is detected in the muscle belly of the sternomastoid near the sternal head. Developmental dysplasia of the hip is associated with the condition in approximately 20 percent of cases.
CliniCal Features
122 Vertebral anomalies Diagnosis in the neonatal period is difficult if there is no sternomastoid tumor. Torticollis is corrected in the neonatal period by gentle manipulative stretching by the mother in the opposite direction of the deformity, usually in the supine position, to reduce muscle spasm.
Upper Limb and Shoulder Girdle Anomalies
Key words: Limited shoulder movement with a high scapula - due to incomplete descent of the shoulder girdle in intrauterine life.
An exaggerated pulsation of the high right subclavian artery is the cause of an increased incidence of pseudarthrosis on the right side. The bony connection is quickly achieved by improving the strength of the shoulder girdle.
CleidoCranial dysostosis
Clinical Findings
This absence of pectoral muscles is evident in a newborn baby and the nipple over the absent muscle is often atrophic.
Congenital RadioulnaR SynoStoSiS
In an older child, the shoulder may exhibit excessive rotational movements due to difficulties caused by the restriction of supination of the forearm. The rotational movements can be achieved by osteotomy of the radius in the middle third; the forearm is placed in a neutral position after the correction.
Congenital SynoStoSiS of the elbow
No attempt should be made to divide the synostosis at any age as the results are poor. Where the amount of rotational correction needed is more than 45°, both the radius and ulna are osteotomized.
There are some noticeable differences in the growth of the forearm and arm in children with radial club hand (Fig. 32.3). In some patients with a radial club hand, the elbow is often fixed in extension.
Postoperative wrist function was not compromised in his series, which was in contrast to what many others have observed. Lamb used tendon transfer in some of these surgically treated cases to provide better wrist function.
Those authors in favor of early surgery advised maintaining the correction as the baby grows to achieve a good functional hand. A short ulna is best treated with lengthening following any of the techniques available with the ortofix or Ilizarov method.
Syndactyly
Polydactyly
Longitudinal Deficiency of Thumb as Part of a Syndrome
In the hypoplastic thumb with proper metacarpophalangeal and interphalangeal joint functions, the web between the thumb and index finger is deepened for better thumb function.
Web Contracture between Thumb and Index Finger
Kirner deformity
140 Anomalies of the upper limb and shoulder girdle Treatment is splinting from the neonatal period in extension-abduction. Sometimes after releasing the soft tissues and obtaining extension-abduction of the thumb, the skin is covered by a rotational flap from the dorsum and a full-thickness skin graft.
Etiopathology
Hand and finger effects present at birth .. pleiotropic dominant mutation affecting skeletal, sexual and apocrine-mammary development); an isolated radioulnar defect; seven had split foot and split hand, one of whom had Karsch-Neugebauer syndrome; and eight had isolated split hand with terminal transverse defect in two. In Karsch-Neugebauer syndrome in one of the 61 cases, a 46-year-old woman and three of her six children had both cleft foot and cleft hand and congenital nystagmus, which is said to be a single gene dominant disorder.
In hand reduction malformations as part of a syndrome, secondary features such as heart disease in Holt-Oram syndrome, visual impairment in Karch-Neugebauer syndrome, although initially appearing less obvious, may eventually be a serious problem health. Family history is also important in patients with hand reduction malformations as 13 patients in Pilarski's series had a positive family background.
NeoNatal osteomyelitis
Neonatal Infections
Since the blood supply to the metaphysis of long bones in newborns communicates with the blood supply to the epiphysis, the infection process extends to the epiphysis, resulting in destruction of the epiphysis in many cases, causing major growth disturbances. The spread of infection is rapid due to poor inflammatory reaction and immature immune response in newborns, resulting in gross destruction of metaphysis and epiphysis and also affection of multiple sites.
Radiographic Changes in straight X-ray
Where B-hemolytic Streptococcus was detected in 28 percent (7 patients) of the above series, consisting of group A in one patient and group B in six; other series7 emphasized the importance of group B B hemolytic streptococcal infection in 69 percent of children with osteomyelitis and/or septic arthritis less than two months of age. The hip joint, which is commonly affected, is treated with an abduction splint until the main epiphysis is well within the acetabulum and shows signs of regeneration of the involved epiphysis.
Complications
Acute PsoAs Abscess
This is a rare case, as the occurrence of a psoas abscess in a newborn has not yet been recorded.
GanGliosidosis Type i
Mucolipidosis ii (i-cell disease)
Miscellaneous Conditions
NeoNatal tetaNus
Down SynDrome
Presenting Features
Differential Diagnosis
Newborns and infants with Down syndrome may suffer from transient myeloproliferation syndrome, which mimics congenital leukemia.
About sixty cases have been reported in the literature1,2, of which half of the cases showed the manifestation of gangrene on the first day of life. Embolic manifestation due to thrombi originating from the umbilical veins3 or arteries4 or from the terminal aorta or iliac vessels or from the renal or adrenal veins5 has been attributed as the cause leading to ischemia and decreased perfusion.
Predisposing Conditions
In cases of gangrene occurring in the upper extremity, no definitive cause of thrombi has been identified, except in one case where the thrombus was reported to have occurred in vessels originating from the aortic arch.3 Neonatal gangrene has been reported to have occurred in three born babies. diabetic mothers with a long history of diabetes.1.
Capillary refill decreases in the distal region and the radial or posterior tibial pulse does not become palpable, depending on the area of the disease. Providone-iodine and a fine-mesh bandage with a soft Jones compression bandage help in most cases with the return of capillary refill and heart rate.
The treatment of the condition consists of fasciotomy of the affected part after preoperative application of antibiotics, preferably on the first day of life. Split skin grafting is necessary to cover the affected area depending on the condition of the wound.
SyStemic LupuS erythematoSuS
A similar tumor, a neuroblastoma arising from the neural crest, is found in the same situation as above and mimics a Wilms' tumor. 162 Miscellaneous conditions Although the incidence of rhabdomyosarcoma in a neonate has been reported, neonatal incidence of bone tumor has not yet been found in the literature.
Sacrococcygeal teratoMa
Conjoined (SiameSe) TwinS
Subperiosteal clearing of the gluteal iliums helped achieve wide separation of the iliac fragments. To prevent sinus formation, 'O' Vicryl suture material was used to attach the pubic bones.
Caffey’s Disease
The condition is differentiated from ost eomyelitis, Ewing's tumor, metastases from neuroblastoma, trauma, syphilis, hypervitaminosis A. The self-limiting nature of the disease, with onset of all the clinical and radiological features within a very short time, makes the condition different from other conditions mentioned above.
The disease affecting infants within five months of age with inflammatory swelling over the mandible, clavicle, scapula and long legs associated with mild fever and hyperexcitability, elevated leukocyte count and ESR is strongly suggestive of Caffey's disease. The characteristic radiographic features of diaphyseal bone formation with periosteal reaction, following soft tissue swelling, make the suspicion even stronger.
Craniosynostosis
Crouzon syndrome
Pfeiffer syndrome
Severe rhizomelic Type
Other clinical and radiological features are virtually the same as the first type, but of lesser magnitude. A skin condition that may not be as serious as the first type may occasionally require treatment.
Pathologically, endochondral bone formation is not affected at all unlike the first type, but the epiphyseal cartilage shows cysts and also calcification. Some of the children with the above traits may still be born or die in the perinatal period.
Index
Carpenter syndrome 20, 138 Cartilage dysplasias 20, 21 Cause of genetic disorders 15 Central nuclear disease 33, 34 Cephalhematoma 38 Cerebral palsy 36 Cervical spine injury 40 Chondrodysplasia. DDenis-Browne splint 84 Deoxyribonucleic acid 13 Compressed fracture 40 Detection of musculoskeletal abnormalities in neonatal period 9 Development of vertebral bodies 113 Developmental dysplasia of hip 100 Diagnosis of developments.
